EUPSA/ERNICA · Surgical treatment for Hirschsprung’s Disease: An ERNICA animation for parents and families
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Video3 min·Published Dec 2023Older

Surgical treatment for Hirschsprung’s Disease: An ERNICA animation for parents and families

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What the experts said0 expert statements · 19 host summaries
Hirschsprung's disease is also known as agangliosis of the colon
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Surgical removal of the affected part is usually performed 2 to 3 months after diagnosis, when the baby is strong enough and growing well
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Surgery is performed when there are no signs of enterocolitis (inflammation of the bowel)
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The aim of surgery is to remove the aganglionic bowel, which is the affected part of the bowel with no ganglion cells present
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The transition zone should be removed, which is the area of the bowel between the aganglionic part and the part containing a normal amount of ganglion cells (ganglionic bowel)
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The remaining part of the bowel is connected to the anal canal in a pull-through procedure to restore the movement of feces
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During the operation, tissue samples can be taken to confirm the presence of ganglion cells
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The procedure used to be done by open surgery with a large incision in the outside skin
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Nowadays, when possible, the procedure is done transanally (via the anal canal), sometimes assisted by laparoscopy
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Different pull-through techniques include the transanal pull-through technique and the Swenson, Duhamel, and Soave procedures
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The surgical techniques used depend on the child, the length of the affected bowel area, the surgeon's preference, and hospital resources
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Preservation of the anal canal is crucial in order to maintain continence
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After surgery, it is important that the new connection between the bowel and anal canal is unobstructed and that surgical wounds heal well
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After surgery, developing babies may experience ongoing difficulties including constipation (difficulty passing feces) and lack of control over bowel movements
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Sometimes support with bowel management is necessary after surgery
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After surgery, individuals with Hirschsprung's disease may remain prone to bowel infections known as enterocolitis
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Structured regular follow-up care by a multidisciplinary team (MDT) of different clinical specialists is essential for babies with Hirschsprung's disease
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Identifying any complications or difficulties early is very important
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Symptoms after corrective surgery can improve as children grow older
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